Is Hirayama a Gq1b disease?

Baslo, Sezin Alpaydın; Erdoğan, Mücahid; Balçık, Zeynep Ezgi; Ozturk, Oya; Ataklı, Dilek

doi:10.1007/s10072-019-03758-x

Cited by 5 publications

(1 citation statement)

References 20 publications

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“…Hematochimic tests showed positive ANA (1:320), which then regressed. IgGq1b were not investigated, but Baslo et al showed the absence of correlation between their presence and HD (29). At the end of 2011, based on neurophysiological and neuroradiological data, the absence of a clear disorder of the motion neuron or a radicular suffering and in her clinical course, a condition like Hirayama's disease was diagnosed, affecting the spinal metamers C7-C8-T1.…”

Section: Patient Presentation Clinical Historymentioning

confidence: 99%

Hirayama Disease: A Case of an Albanian Woman Clinically Stabilized Without Surgery

2020

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Hirayama Disease (HD) is a rare clinical condition that usually affects young people with preference for Asian males. It appears with unilateral distal amyotrophy or asymmetric bilateral amyotrophy of an upper limb which is to refer to an involvement of the spinal metamers C7-C8-T1. A clinical case of a female patient of Albanian nationality is described, with onset of the disease in adulthood and clinical and electrophysiological features suggestive of HD, without any characteristic imaging findings. Clinical investigations, EMG and radiological data facilitated the diagnosis and allowed the exclusion of degenerative forms of the motor neuron and radiculopathies. In this paper, we want to point out that the diagnosis of this pathology should be hypothesized even in the absence of characteristic epidemiological and imaging data.

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Section: Patient Presentation Clinical Historymentioning

confidence: 99%

Hirayama Disease: A Case of an Albanian Woman Clinically Stabilized Without Surgery

2020

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Rare Neurologic Diseases and Neurological Sciences: a report for the celebration of the 2020 Rare Diseases Day

Federico

2020

Neurol Sci

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Hirayama Disease: Review on Pathophysiology, Clinical Features, Diagnosis and Treatment

Gomathy¹,

Agarwal²,

Garg³

et al. 2022

US Neurology

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Hirayama disease is a relatively unusual cause of cervical myelopathy characterized by uni- or bilateral asymmetric weakness, fasciculations and atrophy of the distal upper limbs, mostly seen in young males. The insidious weakness progresses over 3–5 years, after which it stops. Hirayama disease results from the abnormal anterior shifting of the posterior cervical dura that occurs during cervical flexion, leading to cord impingement. Electrophysiologic studies reveal denervation of C7–T1 myotomes and absence of sensory involvement. Dynamic magnetic resonance imaging of the cervical spine is essential in confirming the diagnosis. Treatment is aimed at primarily avoiding neck flexion, which is achieved with the help of a cervical brace; however, progressive disease calls for surgical intervention. Although the disease is self-limiting, it causes many functional impairments in affected individuals. In this review, we describe the epidemiology, pathophysiology, clinical manifestations, imaging characteristics, electrophysiologic findings, differential diagnoses and updates in the treatment of Hirayama disease. This review also aims to improve the awareness of this disease among clinicians, enabling early suspicion, diagnosis and management.

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Is Hirayama a Gq1b disease?

Cited by 5 publications

References 20 publications

Hirayama Disease: A Case of an Albanian Woman Clinically Stabilized Without Surgery

Hirayama Disease: A Case of an Albanian Woman Clinically Stabilized Without Surgery

Rare Neurologic Diseases and Neurological Sciences: a report for the celebration of the 2020 Rare Diseases Day

Hirayama Disease: Review on Pathophysiology, Clinical Features, Diagnosis and Treatment

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